68 May-Hegglin Anomaly

Michelle To and Valentin Villatoro

PBS Key Features:1-4

Graunulocyte and Monoyte cytoplasms contain large basophilic inclusions that resemble Dohle bodies but are much larger and elongated. Inclusions are composed of precipitated myosin heavy chains.

 

Giant platelets and thrombocytopenia are also associated with this anomaly.

 

Cause:1

MYH9 gene mutation

 

Inheritance Pattern:1,5

Autosomal dominant

 

Clinical Significance:1-3,5

May-Hegglin anomaly is a platelet disorder that can cause mild bleeding tendencies but majority of patients are asymptomatic. Degree of bleeding is correlated to the degree of thrombocytopenia. Leukocyte function is unaffected.

 

CBC:1,5

Variable thrombocytopenia


References:

1. Manonneaux S. Nonmalignant leukocyte disorders. In: Rodak’s hematology clinical applications and principles. 5th ed. St. Louis, Missouri: Saunders; 2015. p. 475-97.

2. Harmening DM, Marty J, Strauss RG. Cell biology, disorders of neutrophils, infectious mononucleosis, and reactive lymphocytosis. In: Clinical hematology and fundamentals of hemostasis. 5th ed. Philadelphia: F.A. Davis Company; 2009. p. 305-30.

3. Turgeon ML. Nonmalignant Disorders of Granulocytes and monocytes. In: Clinical hematology: theory and procedures. 4th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1999. p. 206-16.

4. Bain BJ. Morphology of blood cells. In: Blood cells: a practical guide [Internet]. 5th ed. Chichester, UK: John Wiley & Sons, Ltd; 2015 [cited 2018 Jul 10]: 67-185. Available from: http://doi.wiley.com/10.1002/9781118817322

5. Landis-Piwowar K. Nonmalignant disorders of leukocytes: granulocytes and monocytes. In: Clinical laboratory hematology. 3rd ed. New Jersey: Pearson; 2015. p. 388-407.

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May-Hegglin Anomaly by Michelle To and Valentin Villatoro is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License, except where otherwise noted.

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